1. The Hong Kong Disease – Management Updates
JHSGR 17/5/2008
The Hong Kong Disease – Management Updates
Dr. YF Yeung
Department of Surgery
Prince of Wales Hospital

2. The Hong Kong Disease
SARS

3. The Hong Kong Disease Recurrent Pyogenic Cholangitis (RPC)
Oriental cholangitis
Oriental cholangiohepatitis
Intrahepatic pigmented
calculus disease

4. Recurrent Pyogenic Cholangitis
Cook in 1954
Repeated primary biliary infection
Pus-forming bacteria
Multiple stones and strictures in the biliary tree
Pathogenesis not well understood nowadays
Calcium bilirubinate stones within extra- and intraheptic biliary ducts

5. Aetiology ? Oriental diet ? Poor environmental hygiene
Low saturated fat: biliary stasis
Low protein diet: increased formation of calcium bilirubinate stones
? Poor environmental hygiene
Recurrent enteric infection and portal bacteraemia
? Clonorchis sinensis and Ascaris lumbricoids

6. Epidemiology Predominantly lower socio-economic class and rural areas
Male = Female
Peak age incidence: 3rd to 4th decades
Overall incidence is decreasing in East Asia
HK experience
: 30 patients / year
: 22.8 patients / year
Lo et al. HKMJ 1997
Increasing incidence in the West due to Asian immigrants

7. Why intervention important?
Complications of RPC
Recurrent cholangitis
Liver abscess
Parenchyma atrophy
Biliary cirrhosis
Cholangiocarcinoma

8. Imaging Features ERCP Truncated tree sign Ductal ectasia
Abrupt tapering
Arrow head appearance

9. Imaging Features Percutaneous Transhepatic Cholangiography
Severe stricture
Dilated ducts
Multiple filling defects

10. Imaging Features
MRCP
Dilated ducts
Strictures
Filling defects

11. Imaging Features CT Hepatolithiasis Parenchymal atrophy
Obliterated portal vein

12. Management - Multidisciplinary
Acute episode
Control of biliary sepsis
Drainage +/- extraction of stones
ERCP
PTC
Definitive treatment
Correction of anatomic abnormalities/ sources of chronic infections

13. Definitive Management
Surgical
ECBD
Liver Resection
Drainage Procedure
Endoscopic
Percutaneous Transhepatic Cholangioscopic Lithotripsy (PTCL)
“mother-baby” endoscope system

14. Hepatectomy Indications Stones localized in unilateral lobe
Bile duct stricture
Atrophy of affected segments/ lobe
Suspected cholangiocarcinoma
Failed / recurrent disease after non-operative treatment

15. Hepatectomy Series n Mortality Morbidity Stone Clearance Cholangio-CA
FU (mth)
Recur-rence
Chen
2004
103 2%
28%
98%
10% 56
7.8%
Cheung
2005 52
3.8%
33.3% 58
13.3%
(5 yrs)
Uchiyama 2007 38 0%
23.7%
100%
7.9%
108
13.9%
Lee
2007
123
1.6%
92.7%
2.4%
40.3
5.7%

16. Drainage Procedure Principle Indications Eliminate biliary stasis
Newly formed stones can pass unimpeded into the bowel
Indications
Extrahepatic ductal stones
Extrahepatic biliary stricture
Grossly dilated common duct with problem of bile stasis

17. Drainage Procedure Choledochoduodenostomy (CD)
Sump syndrome
Ascending cholangitis
High risk of stasis
Hepaticojejunostomy (HJ)
Hinder post-operative choledochoscopic removal of residual stones
Hepaticocutaneous jejunostomy with a stoma for easy access
Possible complications: fistula, infection, parastomal hernia, early stoma closure
Sphincteroplasty
Parilla P et al. BJS 1991
Rat P et al. Hepatogastroenterology 1993
Huang et al. Am J Gastroenterol 2003

18. Is Drainage Procedure a MUST after hepatectomy?

19. Intra-op bleeding OT time Residual stone Post-op cholangitis
World J Gastroenterololgy 2006
Intra-op bleeding
OT time
Residual stone
Post-op cholangitis
Liver resection(76)
500ml
282min
18.4%
22% HJ
ECBD (47)
300ml
226min
23.4%
27%
314 patients
Liver resection (85)
189min
21.2%
8.2%
T-tube
ECBD (106)
150ml
166min
34%
35.7%

20. Concluded indications for HJ
World J Gastroenterololgy 2006
Median FU 7.6 years (2-12)
Concluded indications for HJ
Hepatolithiasis complicated with extrahepatic ducts or its second branches stricture
Hepatolithiasis with congenital bile duct dilatation in which the dilated bile duct should be resected
Dysfunction of the papilla of Vater

21. Percutaneous Transhepatic Cholangioscopic Lithotripsy (PTCL)
Indications
Stones distributed in multiple segments
Previous biliary surgery
Poor surgical risk
Refuse surgery

22. PTCL Causes of incomplete stone clearance
Biliary stricture
Bile duct angulation
Muddy stones with sludge
Peripheral stone distribution
Biliary stricture is the major determinant for recurrence

23. PTCL Series N Mortality Morbidity Stone clearance Recurrence
Mean FU (mths)
Huang
2003
245
0.8
1.6%
85.3%
63.2%
209
Cheung 79
7.6%
76.8%
30%
37.3
Chen
2005 74 3%
82%
59%
121

24. J Am Coll Surg 1999
Morbidity
Mortality
Stone clearance
5 yr recurrence
10 yr recurrence
Hepatec
-tomy(26)
38.5%
3.8%
96.2%
5.6%
16.0%
54 patients
PTCL (28)
21.4%
3.6%
96.4%
31.5%
54.3%

25. Our Experience on Hepatectomy for RPC
Series N
Mortality
Morbidity
Stone
Clearance
Cholangio-CA
FU (mth)
Recur
rence
PWH 66
36.4%
93.9%
6.1%
42.7
12.9%
Chen
2004
103 2%
28%
98%
10% 56
7.8%
Cheung
2005 52
3.8%
33.3% 58
13.3%
(5 yrs)
Uchiyama 2007 38 0%
23.7%
100%
7.9%
108
13.9%
Lee
2007
123
1.6%
92.7%
2.4%
40.3
5.7%

26. Conclusion RPC is not “dead” in Hong Kong
Health care burden in HK for the recurrent nature of the disease
Management should be of multidisciplinary approach and tailored to individual patient
Hepatectomy is safe and effective